Patients to DHHS: Fix the Broken ME/CFS Case Definitions NOW!

On May 12 - International Awareness Day for ME/CFS/FM/MCS/etc - Phoenix Rising joined with 8 other US ME/CFS patient organizations and 26 independent patient advocates to call on the Department of Health and Human Services (DHHS) to finally fix the problem of the many and diverse case definitions associated with our disease. In a letter to Secretary Sebelius, Dr Howard Koh, Dr Thomas Frieden and Dr Francis Collins, we explained our concerns about the current definition activities of the DHHS in relation to "Chronic Fatigue Syndrome", and listed the steps we believe must be taken to rectify the situation.

For those of you who wish to sign this letter and become a part of this important initiative, we will provide a mechanism to do that within a few weeks and will send out additional information at that time.

Why have we written this letter?

Of all the issues that we face today, the one issue that has created the most problems is the diverse case definitions associated with our disease. This single issue has severely impacted research, drug development and clinical care and misled the medical community on the very nature of this devastating disease, causing many doctors to not believe that their patients are really sick. Until this issue is addressed, patients will continue to pay the price. This must stop now.

Today, the CDC states that there are at least 5 different definitions for “CFS”. Three of these definitions - the Canadian Consensus Criteria, the ME International Consensus Criteria and the Pediatric Criteria - require hallmark criteria like PEM/PENE and neurological, immunological and energy production impairments. Unfortunately, two of the most commonly used definitions, Fukuda and Oxford, do not require these hallmark criteria. In fact, Oxford only requires 6 months of disabling fatigue - no other symptom - and allows primary psychiatric disorder.

The result? Myalgic encephalomyelitis, the disease seen in outbreaks throughout the twentieth century and recognized by the World Health Organization in 1969, has disappeared, and in its place we are left with “CFS”, an amorphous umbrella of unrelated fatiguing conditions including, according to the literature, depression, deconditioning, medically unexplained chronic fatigue, and for some researchers and clinicians, fatigue due to “excessive rest” or “false illness beliefs”. In clinical practice, the diagnosis of CFS is given to a heterogeneous mix of patients – those with ME, those with the varied fatiguing conditions listed above, and those who were misdiagnosed or whose doctors use the diagnosis of CFS as a catch-all for unexplained fatigue. And in 2012, an American Family Physician article proclaimed that Oxford and Fukuda are the appropriate definitions for “CFS” and further stated: “[CFS] patients with poor social adjustment, a strong belief in an organic cause for fatigue, or some sort of sickness benefit (i.e., financial incentive) tend to have worse responses to [cognitive behavioral] therapy.”

Exactly what disease are we talking about here?

Patients have paid dearly for the proliferation of these overly broad and non-specific definitions – bedbound or homebound, unable to work or take care of their families, suffering for 10, 20, 30 or more years from the myriad symptoms that plague their bodies, unable to get adequate medical care and ultimately more likely to die prematurely from cancer, cardiovascular disease and suicide.

As Dr. Carruthers stated in the ME International Consensus Criteria, “Research on other fatiguing illnesses, such as cancer and multiple sclerosis, is done on patients who have those diseases. There is a current, urgent need for ME research using patients who actually have ME.” We must have a disease appropriate definition for ME that is separate and distinct from all the other unrelated conditions encompassed by the overly broad, fatigue-focused “CFS” definitions.

What are we asking for?

Our letter to the DHHS asks them to:

Adopt a disease-appropriate case definition for ME now, utilizing the Canadian Consensus Criteria as recommended by DHHS’ own advisory committee CFSAC, and train doctors with appropriate medical guidance.

Stop using the terms “CFS” and “Chronic Fatigue Syndrome” along with the non-specific definitions like Fukuda and Oxford and the medical education material based on these definitions.

Manage the adoption of the Canadian Consensus Criteria to ensure that insurance and disability do not lapse and that no patients fall through the cracks.

Fully engage ME stakeholders in the planning and execution of the adoption of the Canadian Consensus Criteria.

Is this the right thing to do?

You may ask whether we really know enough about the disease or whether we need more study before we change definitions. Certainly, with more study, we can better operationalize the definition and validate biomarkers to make patient diagnostics easier. But in the meantime, we know that PEM/PENE is a hallmark symptom that reflects a distinctive biological pathology and we must utilize a disease definition that requires that symptom.

Some of you may prefer the ME International Consensus Criteria over the Canadian Consensus Criteria. The ME-ICC certainly has some excellent features. But practically, the Canadian Consensus Criteria has been used clinically and in research for a decade. Studies have been done with it. The U.S. government has posted the IACFS/ME Primer, based on the Canadian Consensus Criteria, on DHHS’ Guidelines.Gov. This is more likely to be acceptable to DHHS and is a reasonable first step, especially when considered against the alternative of continuing to use Fukuda while more study is done.

What about dropping the name “CFS”? You may be concerned that this means we will lose the literature base that has provided insights into the pathology of ME. Admittedly, some of the best articles used the term “CFS”. And so do some of the worst. The point is that the literature base is a mess because multiple diverse and unrelated definitions have inexplicably been allowed to use the very same name for years. We all should stop using the term “CFS” because it no longer has any real meaning.

Finally, what about the name ME? Does it really describe the disease? Is there a better name? That is a question that science will need to decide over time, something that has happened in many other diseases. But what is clear is that “chronic fatigue syndrome” will never be an appropriate name and should never have been established as the alternative or synonym for ME.

Patients have borne the brunt of the failure to address the definitional issues for the last thirty years. We cannot wait for more study to finally stop the harm being done to patients, especially given that more study with non-specific definitions will only perpetuate the problem. The time to address this problem is now.

Questions and Answers

We realize that patients, carers and advocates may have a number of questions about this initiative, and we hope that the following questions and answers will address any concerns you may have.

1. We can not abandon the patients that have been incorrectly given a “CFS” diagnosis.

This is very true. It is critical that implementation of this change is carefully managed so that these patients are re-evaluated and given a correct diagnosis. If unexplained conditions remain, it will be necessary to perform the studies needed to understand these conditions and establish more appropriate names and definitions.

2. We can not afford to have our disability or insurance impacted.

Yes, this is very important. It will be important to have a carefully thought out implementation plan that manages this to ensure that patients do not lose disability or insurance benefits.

3. The vast majority of the 6000 articles in the literature use the name “CFS”, not “ME. If we stop using the name “CFS”, we will lose all that literature.

Currently, when the search term myalgic encephalomyelitis is used, the CFS literature is returned. This will not change. But that literature base contains both articles relevant to ME and also a significant number of articles about “CFS” and child abuse, false illness beliefs, deconditioning, etc. This creates significant confusion for anyone trying to use that literature. For that reason, the non-specific term “CFS” should be abandoned by the U.S. and more specific terms like ME used going forward.

4. We have more important issues to deal with such as funding, and attracting new doctors and researchers.

It is critical that we have more funding but if we don’t fix the definition issue first, we will continue to study the wrong disease and have progress impeded by poor definitions. The resultant confusion will make it difficult to attract young researchers and doctors who will not see career opportunity in “CFS”.

5. Research centers have recently been established and if we stop using the name “CFS” we will confuse our donors.

It is true that a number of research institutes have recently been opened and some of them use the term “”CFS” or even “CF”. But the donors to these institutes today have a personal connection to the disease. They will continue to fund. Attracting additional funders, however, will be negatively impacted by the confusion around the disease. The sooner we can resolve this issue, the better in the long run.

6. CFS biobanks have been established using Fukuda and we don’t want to lose those samples.

The biobanks that have only been characterized by the Fukuda definition could contain a mix of patients with the hallmark criteria of ME and those who do not have these hallmark criteria. Using these mixed samples will continue to confound research. It is important that we have a well-characterized set of samples in the biobank and know which samples are from ME patients.

7. ME may not be the right name. Shouldn’t we wait for the science to figure out what the right name is?

It is possible that with further study, we will determine a better name than ME and it will naturally evolve. But ME, adopted by the World Health Organization in 1969, is the best placeholder until that time and avoids the serious issues caused by the use of the term “CFS”.

8. The best course is to tighten up the “CFS” definition, not get rid of it. Then we can keep the literature base, the biobanks, etc.

There are two problems with this approach. First is the long history of the term “CFS”, which is non-specific and now widely associated with diverse conditions, especially including psychiatric issues. This has severely tainted the term and made it clinically meaningless. Second, the term “CFS” is used for those studying patients that meet Oxford criteria (essentially chronic fatigue) and we have little control over that continued usage.

9. Lenny Jason recently published a paper that reports that the ME-ICC and the Canadian Consensus Criteria include more psychiatric co-morbidities than the Fukuda and recommends that more study be done. Does that mean we should wait to recommend any criteria until then?

Dr. Jason’s paper did find that the ME-ICC found more psychiatric co-morbidity than Fukuda. But Dr. Jason acknowledged the need for more study because this one used a questionnaire designed for Fukuda CFS, and that they were unable to assess one of the key ME-ICC criteria because data on this criteria was not available. Further, the study did not look at homebound or bedbound patients.

But what is also significant in Dr. Jason’s study is that ME-ICC identified a much tighter group of patients (39 compared to 113 for Fukuda) with more of the functional impairments and physical, mental and cognitive problems seen in ME-ICC patients than in those meeting the Fukuda criteria.

Clearly additional study is needed to operationalize the definition and to improve how it characterizes the disease, especially around subtypes. But continuing to use the 19-year-old consensus-driven Fukuda definition - which is also not operationalized and does not describe subtypes - in the meantime is not going to advance that knowledge and will only continue to hurt patients.

The Canadian Consensus Criteria has been used clinically and in research for over 10 years and better represents the disease. Using the CCC now will allow us to begin to make forward progress in research and identifying treatments, and begin to address the disbelief in the medical community.

10. Is this the same thing as the Name Change initiative?

No. This is first and foremost about the definition being used – adopting a definition that effectively describes the disease and stopping the use of the definition – and name – that have created so much confusion and so many problems.

11. Why CCC and not ME-ICC?

The CCC has been used clinically and in a number of studies, providing the experiential foundation for its use. It is expected that as additional data is obtained, this definition will evolve. This must be done in partnership with the experts who developed the ME-ICC and the CCC.

We hope and believe that this initiative will be welcomed by the majority of the patient community, and we hope that the questions above have addressed any concerns. Of course there is always room for debate over details, but very few if any of us are happy with the existing definitional mess, and this letter represents a consensus amongst 9 patient organizations and 26 independent advocates on the best path towards change. As such, we encourage the community to get behind this initiative and seize this opportunity to resolve the problem of the broken case definitions used for our disease.

Phoenix Rising is a registered 501 c.(3) non profit. We support ME/CFS and NEID patients through rigorous reporting, reliable information, effective advocacy and the provision of online services which empower patients and help them to cope with their isolation.

There are many ways you can help Phoenix Rising to continue its work. You can even donate significant sums, at no cost to yourself, as you shop online! To find out more, visit Phoenix Rising’s Donate page by clicking the button below.

I'm sure there are areas where people might disagree but like just about every complex issue, I think it's best to focus on the big wins - huge wins - we'll get if we get behind this and not on side-issues.

The use of the Oxford criteria here in the UK has been catastrophic - catastrophic - and we've got to get back to a tighter defintion.

Well done to those who have worked on this. We're immensely stronger when we stand together and speak as one voice. I'm glad to see our advocates and advocacy organisations working together on this important issue. We need to see more of this.

The placement of #2 in the list is going to raise many cynical eyebrows. It's true, but considering that
i) that people are scamming off disability is a common trope
ii) it got that way bc there is a subculture which proudly does exactly that. e.g., a co-worker of mine who fell down stairs at his house while drunk and arranged to get it written up as a work injury, etc etc
iii) thus primed to see a zebra in the shadows, people unfamiliar with our disease will see a zebra in the shadows.

I think we have to be very careful to lay out information first. It then becomes clear, or at least visible that outcomes are much worse when we are struggling with insurance/disability to maintain a shadow of former income. I might have been able to get back to work had the insurance been working with me instead of against me; having a zero income, being homeless etc appears to have set off a permanent entrenchment of the disease.

I agree with this proposal, at least in general. I have yet to give it a detailed analysis. However I can see the counter-argument: but the CDC are already working on this! They have made progress! You are not prepared to give them time to finish? We have to be prepared for these kinds of arguments.

Has anyone considered a broader petition to have this request acted on? Something everyone can sign?

The placement of #2 in the list is going to raise many cynical eyebrows.

Click to expand...

I think the concern isn't that CF patients will lose related disability/insurance benefits, but rather that the change of name might result in bureaucratic confusion where patients newly diagnosed with "ME" won't have a recognized disease, and/or patients with an old diagnosis of "CFS" will have a disease which no longer exists.

Given the propensity for benefits agencies and especially insurance companies to bungle things in a way which is profitable for themselves, a name/definition/classification change needs to be carefully planned, implemented, and monitored.

Has anyone considered a broader petition to have this request acted on? Something everyone can sign?

Click to expand...

Yes, that's under consideration, there has been quite a bit of discussion about that. I think it's fairly likely there will be one in the fulness of time, but for now, this thread is a good place for our members to indicate their support and express their views on the subject in general.

Actually say ME/CFS here in the sticks everybody's eyes glaze over, or much feet shuffling, A proper name more descriptive since we know the essential pathologies - Immune system, Endocrine system, Neurological system. NEI (not too catchy) perhaps, and ME thoroughly discredited by the psyche lobby here in the UK. As far as I'm concerned the Immune system crashed, research findings confirm - why - this is whatever the viral/bacterial overload compromised. A bit of Sherlock Holmes perhaps but the essential is to my mind something overcoming the Immune system.

I personally support this letter, but I know, from talking to various patients over a number of years, that this issue raises major concerns for some CFS patients. Some CFS patients have had a CFS diagnosis for a long time, are comfortable with it, and familiar with it. They and their doctors may not be familiar with the term 'ME', may not be familiar with the differences between CFS and ME, or may not be familiar with the details of the science or the politics that has played out in relation to this disease over the decades. Or some patients simply do not agree that ME is a discrete entity, and are of the opinion that not enough science has been carried out to scientifically separate CFS from ME. And then of course, some patients currently diagnosed with CFS, may not experience Post Exertional Malaise, or neurological symptoms etc.

I think some patients will find it threatening to have the term 'CFS' withdrawn, and maybe rather worried and even frightened about having such a change implemented. Some CFS patients might worry: "Where does it leave me if I can no longer have a CFS diagnosis?"

So I appreciate the leadership in this area and, in my opinion, it's certainly an essential issue that needs to be addressed, as an urgent priority. But, in my opinion, any clinical changes need to be designed and implemented with much thought, and great care and sensitivity, making sure that no patients feel frightened, neglected or abandoned as a result of any changes. If great care is not taken then there may even be a backlash against this move, from some very worried CFS patients, which the government authorities will find confusing. A patient backlash against an insensitive implementation of new policy could be counter-productive in terms of achieving the desired long-term goals.

So this project has my personal support but as long as it is implemented with care, compassion, consideration, and sensitivity towards all patients.

What I think can be safely implemented immediately, without any consultation necessary, is that all government-funded research should be expected to use a CCC cohort, alongside any other cohorts that the researcher requires.

Broadly speaking, I agree with this and I'm pleased to see it happening. Am I right to suspect that this group of organizations that are collaborating together, is growing and reaching out to other like-minded organizations?

And is there a method in place by which new organizations can add their signature to this letter? And subsequent collaborative efforts like this?

I do think the name is less important than the definition and the actions. The name ME is already co-opted by psychobabble. Most doctors probably think or suspect its hysteria/somatization/functional, especially in the UK. The trashing and distortion of the label ME began 18 years before the term CFS was created. The term CFS was simply a further step.

Getting a good definition in use in both research and clinical practice is thus the real goal until the science advances, and doing it in a way that causes minimal problems. Of course those definition all happen to be ME definitions. What a name change does though is send a message, and one that might be very worthwhile to get out there.

I wonder if it not too confusing for the average Doc, unable to rise above (like in their textbooks) - a combination of symptoms - they are educated to one simple "organ" dysfunction only. Well I must say all my (failed) investigations (they couldn't understand), it is accepted one has to be rather brighter than your average Doc (including those who choose psychiatry) to enter the fields of neurology, immunology, virology etc.

I personally support this letter, but I know, from talking to various patients over a number of years, that this issue raises major concerns for some CFS patients. Some CFS patients have had a CFS diagnosis for a long time, are comfortable with it, and familiar with it. They and their doctors may not be familiar with the term 'ME', may not be familiar with the differences between CFS and ME, or may not be familiar with the details of the science or the politics that has played out in relation to this disease over the decades. Or some patients simply do not agree that ME is a discrete entity, and are of the opinion that not enough science has been carried out to scientifically separate CFS from ME. And then of course, some patients currently diagnosed with CFS, may not experience Post Exertional Malaise, or neurological symptoms etc.

I think some patients will find it threatening to have the term 'CFS' withdrawn, and maybe rather worried and even frightened about having such a change implemented. Some CFS patients might worry: "Where does it leave me if I can no longer have a CFS diagnosis?"

So I appreciate the leadership in this area and, in my opinion, it's certainly an essential issue that needs to be addressed, as an urgent priority. But, in my opinion, any clinical changes need to be designed and implemented with much thought, and great care and sensitivity, making sure that no patients feel frightened, neglected or abandoned as a result of any changes. If great care isn't taken then there may even be a backlash against this move, from some very worried CFS patients, which the government authorities will find confusing. A patient backlash against an insensitive implementation of new policy could be counter-productive in terms of achieving the desired long-term goals.

So this project has my personal support but as long as it is implemented with care, compassion, consideration, and sensitivity towards all patients.

What I think can be safely implemented immediately, without any consultation necessary, is that all government-funded research should be expected to use a CCC cohort, alongside any other cohorts that the researcher requires.

Click to expand...

We're very mindful of those concerns, Bob, hence the lengthy explanation of the letter and the questions and answers. You're absolutely right to emphasize that the implementation requires a great deal of care to address the concerns you've mentioned, and we will be taking great care over that. Phoenix Rising, in particular, is very mindful that the objectives of the non-profit cover "ME/CFS", which I take to mean that we have an obligation to defend the interests of everybody who currently has an ME, CFS, or ME/CFS diagnosis. We're certainly not prepared to abandon "CFS but not ME" patients and leave them with no diagnosis and no medical cover. As far as clinical application goes, there will have to be a plan as to what happens to those patients.

But as Julia Newton's research found, something like 40% of ME/CFS patients (in the UK at least) actually have another undiagnosed illness. That's just one study, but even in that study the true figure could be even higher of course, and we know from our own experience that missed diagnosis in the ME/CFS population is a huge problem. So if that's typical of the situation of patients under the "CFS" umbrella, then how can we ever hope to get effective research results from a mixed cohort like that? While such mixed cohorts remain, confusion and contradictory research results are inevitable. So I agree with you: the mandatory use of CCC for research that purports to study "ME" is the first and most important focus. Going beyond that, though, to the clinical definition, it's just as unhelpful to allow for an umbrella-type "CFS" diagnosis that leaves patients with other (sometimes life-threatening) conditions in limbo. That does no service to those patients either. It's long past time to throw away that waste-basket...

Broadly speaking, I agree with this and I'm pleased to see it happening. Am I right to suspect that this group of organizations that are collaborating together, is growing and reaching out to other like-minded organizations?

Click to expand...

I think that's a reasonable assumption. It's a fairly loose 'coalition' at the moment but I expect it to grow and take shape, perhaps a somewhat more formal structure, in the course of the next year or two. Time will tell...

And is there a method in place by which new organizations can add their signature to this letter?

Click to expand...

As I mentioned above, mechanisms for some kind of petition are under discussion, but this particular letter has been written and delivered so in that sense it's completed. I guess anybody who wants to is free to contact the recipients of the letter and express their support for it...

I do think the name is less important than the definition and the actions. The name ME is already co-opted by psychobabble. Most doctors probably think or suspect its hysteria/somatization/functional, especially in the UK. The trashing and distortion of the label ME began 18 years before the term CFS was created. The term CFS was simply a further step.

Getting a good definition in use in both research and clinical practice is thus the real goal until the science advances, and doing it in a way that causes minimal problems. Of course those definition all happen to be ME definitions. What a name change does though is send a message, and one that might be very worthwhile to get out there.

Click to expand...

Spot on Alex. I don't think it's a good idea to get too hung up on the name: the important issue here is the case definition behind the name, and that's what the letter aims to focus on.

You've put it so succinctly here that I'll repeat this sentence from your quote, for emphasis:

Getting a good definition in use in both research and clinical practice is thus the real goal until the science advances, and doing it in a way that causes minimal problems. Of course those definitions all happen to be ME definitions.

Getting a good definition in use in both research and clinical practice is thus the real goal until the science advances, and doing it in a way that causes minimal problems. Of course those definitions all happen to be ME definitions.

Click to expand...

Alex and Mark - I agree - the name is nothing without getting a definition that describes the hallmark symptoms of the disease. Whatever name is used, if it becomes associated with the diversity of definitions associated with CFS today, its going to cause confusion.

And is there a method in place by which new organizations can add their signature to this letter?

Click to expand...

Snowathlete and Mark - The intent is to provide a mechanism for others to sign onto this letter and then we will resend the letter with the additional signatures. We just need to figure out the easiest/best way to do that. Will let you know as soon as its in place.

But as Julia Newton's research found, something like 40% of ME/CFS patients (in the UK at least) actually have another undiagnosed illness.

Click to expand...

That's interesting and seems to be reinforced by some other studies. There's also the large Nacul study done in England that found a prevalence for Canadian Consensus Criteria patients that was roughly half of Fukuda CFS patients. And Maes' examined Fukuda CFS patients and found that roughly half had PEM while the others did not. Finally, a recent study by Vincent found that only 36% of CFS patients examined had PEM although she said it could be a methodological issue.

The challenge is that there is no way to know what that 50% is once you pull of patients that meet CCC - especially if Oxford was used. Some probably have a known medical disease and have been misdiagnosed. But others may have some condition that has not been effectively studied yet. They need to be correctly diagnosed or studied further for the disease they have

That's interesting and seems to be reinforced by some other studies...The challenge is that there is no way to know what that 50% is once you pull of patients that meet CCC - especially if Oxford was used. Some probably have a known medical disease and have been misdiagnosed. But others may have some condition that has not been effectively studied yet. They need to be correctly diagnosed or studied further for the disease they have

Can anyone comment on the process? Who had input? Were any medical or policy experts consulted? What does the endorsement by Phoenix Rising mean?

Click to expand...

medfeb can describe the process better than I can, because Phoenix Rising only got involved in the drafting process partway through. As far as I understand it, the concept and then the letter was discussed extensively amongst the signatories over a period of some months, revised several times in response to comments, and participants then decided whether to sign the final draft.

The Phoenix Rising board decided (unanimously) to sign the letter. Based on previous discussions on the forums over the last few years, I felt that most of our members agree with the objectives set out in the letter (for example, a long thread discussing case definitions some time ago found almost unanimous support for campaigning for the use of the CCC for research, amongst members with a variety of different opinions on other matters), and many members have said in the past that they want Phoenix Rising and other ME/CFS organizations to work together and speak with a strong voice. So my opinion was, and is, that most of our members would want Phoenix Rising to sign this letter.

However, the board is mindful that Phoenix Rising does not yet have 'democratic' processes in place for board membership or advocacy decisions, and as we have stated before, we are committed to putting such processes in place: that is what the board wants to do. Believe it or not, we actually don't want to take these decisions, we want to enable our members to do so. In the future, the aim is to establish an Advocacy Team, and I expect that in future it will be that team (or a subgroup of it) who will take part in such discussions and make decisions on whether to sign any letters presented to us. Moving to that model is not as simple or straightforward as some might imagine, but we are determined to do that, and we look forward to Phoenix Rising co-operating closely with other organizations and individual advocates in the future, as we have done here. I don't want to sidetrack this thread with that discussion of PR internal processes, though; that's something we're working on and we should focus on the letter itself here.

Meanwhile, I believe that we have taken a decision that most of our members will agree with, and I hope that our members will sign the letter when a mechanism to do that becomes available in a few weeks time.